Treatment of Achalasia
For types I and II achalasia, pneumatic dilation (PD), laparoscopic Heller myotomy (LHM), or peroral endoscopic myotomy (POEM) are all effective first-line treatments with the choice based on local expertise and patient factors; however, for type III achalasia, POEM is the preferred treatment due to its ability to perform extended myotomy tailored to the proximal extent of spasm. 1
Diagnostic Workup Required Before Treatment
Before initiating therapy, comprehensive phenotyping is essential:
- High-resolution manometry (HRM) is the gold standard for diagnosis and must define the Chicago Classification subtype (I, II, or III), as this critically determines optimal treatment selection 1
- Esophagogastroduodenoscopy (EGD) with careful retroflexed examination to exclude pseudoachalasia and assess for retained secretions, puckered gastroesophageal junction 1
- Timed barium esophagram to assess structural changes, degree of dilation, sigmoid deformation, and confirm outflow obstruction 1
- Functional luminal impedance planimetry (FLIP) as an adjunct to confirm impaired EGJ opening when HRM findings are equivocal 1
Treatment Algorithm by Achalasia Subtype
Type I and II Achalasia
Three equally effective options exist, with selection based on specific patient factors: 1
Pneumatic Dilation (PD):
Laparoscopic Heller Myotomy (LHM) with Fundoplication:
POEM:
- Superior to PD and noninferior to LHM in multicenter RCTs 1
- When compared appropriately (POEM + PPIs vs. LHM + fundoplication), either approach is conditionally recommended 3
- Critical caveat: Post-POEM patients are high risk for reflux esophagitis and require indefinite PPI therapy and/or surveillance endoscopy 2
- Should only be performed by experienced physicians in high-volume centers (20-40 procedures needed for competence) 2
Type III Achalasia (Spastic Achalasia)
POEM is the preferred treatment because it allows unlimited proximal extension of myotomy to address the full extent of esophageal body spasm, which is critical for symptom relief in this subtype 1, 2, 3
- The myotomy length should be calibrated to the proximal extent of spastic contractions, averaging 17.2 cm 2
- Laparoscopic approaches cannot achieve adequate proximal myotomy extension 1
- Weighted pooled response rate of 92% (95% CI: 84%-96%) for POEM in type III achalasia 2
Special Considerations and Pitfalls
End-Stage Disease
- Patients with severe esophageal dilation, sigmoid deformation, or epiphrenic diverticulum present challenges 2
- Insufficient data exists on POEM efficacy for advanced dilation and sigmoidization 2
- One report suggests doubling of adverse events with POEM in sigmoid esophagus 2
- International guidelines recommend consideration of laparoscopic or endoscopic approaches initially, even in end-stage disease, before esophagectomy 4
Esophagogastric Junction Outflow Obstruction (EGJOO)
- Do not perform POEM based on EGJOO manometric findings alone 1
- EGJOO is not pathognomonic for any diagnosis and requires comprehensive evaluation with symptom correlation 1
- POEM should only be considered case-by-case after less invasive approaches are exhausted 1
Post-Treatment Reflux Management
- All POEM patients require counseling about high reflux risk before the procedure 2
- Up to 58% of patients develop gastroesophageal reflux when systematically studied with pH-metry 2
- Indefinite PPI therapy is typically necessary 2, 3
- Surveillance endoscopy should be considered 2
Failed Primary Treatment
- Success rates for repeat PD or myotomy are lower in previously treated patients compared to treatment-naïve patients 5
- Treatment selection for recurrence is challenging and requires expert evaluation 5
Obsolete or Limited-Use Therapies
- Botulinum toxin injection: Reserved only for patients who are not candidates for definitive treatment due to comorbidities; effect wears off after months and provides progressively less benefit with reinjection 6, 7, 5
- Oral pharmacologic agents: Fallen out of use due to insufficient efficacy and frequent side effects 5